Summary
The neurofibrillary tangles found in the neurons of a case of Steele-Richardson-Olszewski syndrome were studied by electron microscopy. Both the flame-shaped and globose type of tangles were present. The neurofibrillary tangles were composed of bundles of straight tubules measuring approximately 150 Å in diameter. This ultrastructural appearance of the tangles in Steele-Richardson-Olszewski syndrome is different from the appearance of tangles found in other neurological disorders and is probably indicative of intraneuronal accumulation of a new type of fibrous protein.
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References
Anzil, A. P.: Progressive supranuclear palsy. Case report with pathological findings. Acta neuropath. (Berl.)14, 72–76 (1969)
Behrman, S., Carrol, J. D., Janota, I., Mathews, W. B.: Progressive supranuclear palsy. Clinico-pathological study of four cases. Brain92, 663–678 (1969)
Burger, P. C., Vogel, F. S.: The development of the pathologic changes of Alzheimer's disease and senile dementia in patients with Down's syndrome. Amer. J. Path.73, 457–476 (1973)
Constantinidis, J., Tissot, R., DeAjuriaguerra, J.: Dystonie oculofacio-cervicale ou paralysie progressive supranucleaire de Steele-Richardson-Olszewski: Pseudoparalysie du regard, troubles visuospatiaux, pseudodémence, alterations neuronales. Rev. neurol.122, 249–262 (1970)
Hirano, A., Malamud, N., Kurland, L. T.: Parkinsonism-dementia complex, an endemic disease on the island of Guam. II. Pathological features. Brain84, 662–679 (1961)
Hirano, A.: Neurofibrillary changes in conditions related to Alzheimer's disease. In: Alzheimer's disease and related conditions. Ciba Foundation Symposium. G. E. W. Wolstenholme, M. O'Connor (eds.), pp. 185–207. London: J. & A. Churchill Ltd. 1970
Jellinger, K.: Progressive supranuclear palsy (subcortical argyrophilic dystrophy). Acta neuropath. (Berl.)19, 347–352 (1971)
Meyers, K., Dorencamp, D. G., Suzuki, K.: Amyotrophic lateral sclerosis with diffuse neurofibrillary changes. Arch. Neurol. (Chic.)30, 84–89 (1974)
Ohara, P. T.: Electron microscopical study of the brain in Down's syndrome. Brain95, 681–684 (1972)
Olson, M. I., Shaw, Ch.-M.: Presenile dementia and Alzheimer's disease in mongolism. Brain92, 147–156 (1969)
Powell, H. C., London, G. W., Lampert, P. W.: Neurofibrillary tangles in progressive supranuclear palsy. J. Neuropath. exp. Neurol.33, 98–106 (1974)
Schochet, S. S., Jr., Lampert, P. W., Earle, K. M.: Neuronal changes induced by intrathecal vincristine sulfate. J. Neuropath. exp. Neurol.27, 645–658 (1968)
Schochet, S. S., Jr., Lampert, P. W., McCormick, W. F.: Neurofibrillary tangles in Down's syndrome. A light and electron microscopic study. Acta neuropath. (Berl.)23, 342–346 (1973)
Seitelberger, F.: Heterogenous system degeneration. Subcortical argyrophilic dystrophy. Acta neurol.24, 276–284 (1969)
Steele, J. C., Richardson, J. C., Olszewski, J.: Progressive supranuclear palsy. Arch. Neurol. (Chic.)10, 333–359 (1964)
Tellez-Nagel, I., Wiśniewski, H. M.: Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch. Neurol (Chic.)29, 324–327 (1973)
Terry, R. D.: Neuronal fibrous protein in human pathology. J. Neuropath. exp. Neurol.30, 8–19 (1971)
Terry, R. D., Wiśniewski, H. M.: The ultrastructure in the neurofibrillary tangles and the senile plaque. In: Alzheimer's disease and related conditions. Ciba Foundation Symposium. G. E. W. Wolstenholme, M. O'Connor (eds.), pp. 145–165. London: J. & A. Churchill 1970
Wiśniewski, H. M., Terry, R. D., Hirano, A.: Neurofibrillary pathology. J. Neuropath. exp. Neurol.29, 163–176 (1970)
Wiśniewski, H. M., Terry, R. D.: An experimental approach to the morphogenesis of neurofibrillary degeneration and the argyrophilic plaque. In: Alzheimer's disease and related conditions. Ciba Foundation Symposium. G. E. W. Wolstenholme, M. O'Connor (eds.), pp. 223–248. London: J. & A. Churchill 1970
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This investigation received financial support from the World Health Organization.
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Roy, S., Datta, C.K., Hirano, A. et al. Electron microscopic study of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Acta Neuropathol 29, 175–179 (1974). https://doi.org/10.1007/BF00684775
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DOI: https://doi.org/10.1007/BF00684775